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Ten patients with sickle cell (SS) disease from a Jamaican family were found to have unusually high levels of haemoglobin F for this population. Each of them has inherited one sickle cell gene on a chromosome characterized by an arrangement of restriction fragment length polymorphisms (haplotype) which is very rare in the Jamaican population. Genetic analysis of the family suggests that there is a determinant linked to the beta-globin gene cluster, characterized by this haplotype, which is responsible for increased haemoglobin F production in response to anaemia. Interestingly this particular haplotype appears to be common in patients with SS disease in eastern Saudi Arabia in whom a high level of haemoglobin F is the rule rather than the exception. Hence it is possible that this haplotype (++-++) acts as a genetic marker for elevated levels of haemoglobin F in sickle cell disease.

Type

Journal article

Journal

Br J Haematol

Publication Date

1985

Volume

60

Pages

261 - 268

Keywords

Anemia, Sickle Cell/*genetics Female Fetal Hemoglobin/*genetics *Genetic Markers Haploidy Human Jamaica Male Pedigree Saudi Arabia Sickle Cell Trait/*genetics